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恰当与错乱:小儿抗磷脂综合征的识别

Fitting and flailing: recognition of paediatric antiphospholipid syndrome.

作者信息

Freeman H, Patel J, Fernandez D, Sharples P, Ramanan A V

机构信息

Department of Paediatrics, Raigmore Hospital, , Inverness, UK.

出版信息

Arch Dis Child Educ Pract Ed. 2014 Feb;99(1):28-36. doi: 10.1136/archdischild-2012-302404. Epub 2013 Oct 3.

Abstract

Antiphospholipid syndrome (APS) is a systemic autoimmune condition where the presence of antiphospholipid antibodies is thought to predispose to thrombotic events. It is uncommon in the paediatric population, but current diagnostic criteria are based on adult population studies, making assessment of its true paediatric prevalence difficult. We present two cases of paediatric APS, who presented with primary neurological events, and discuss approaches to diagnosis, interpretation of screening investigations, including antinuclear antibodies (ANA), anti-extractable nuclear antigen (ENA) antibodies and lupus anticoagulant. Possible approaches to the management of paediatric APS are discussed.

摘要

抗磷脂综合征(APS)是一种全身性自身免疫性疾病,抗磷脂抗体的存在被认为易引发血栓形成事件。它在儿科人群中并不常见,但目前的诊断标准基于成人研究,这使得评估其在儿科的真实患病率变得困难。我们报告两例患有原发性神经系统事件的儿科抗磷脂综合征病例,并讨论诊断方法、包括抗核抗体(ANA)、抗可提取核抗原(ENA)抗体和狼疮抗凝物在内的筛查检查结果的解读。还讨论了儿科抗磷脂综合征可能的管理方法。

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